Identifying Scleroderma Patients at Risk of Pulmonary Hypertension - US Study Provides Potential Biomarker for the Condition

As many learn when they’re first diagnosed, scleroderma has a variety of connected health issues which drastically differ between patients living with different forms of the condition. To add to this, being diagnosed with a specific type of scleroderma doesn’t guarantee that you’ll experience a uniform set of symptoms – those which severely affect one patient can sometimes never develop in another, despite both having the same sub-type of the disease.

As such, when you’re told that you have scleroderma, it’s common to feel confusion about which symptoms and health problems are most likely to affect you over time, and what treatment you’ll need as a result. This uncertainty can also impact treatment, as clinicians are sometimes unable to intervene with effective medication until symptoms progress enough to become noticeable.

But can we identify patients that are at particular risk of developing certain symptoms before they progress, to limit this uncertainty and give clinicians a better idea of which treatments will be needed? Fortunately, as research progresses, we are moving considerably closer towards the answer becoming ‘yes’! Thanks to research which evaluates the prevalence of various symptoms amongst patients with specific biomarkers like autoantibodies, it will be possible for clinicians to identify those at particular risk of developing related health issues like pulmonary hypertension (PH) and interstitial lung disease (ILD). This means that doctors will be able to detect such complications sooner and start treatment earlier.

The Pittsburgh Scleroderma Center Antibody Study

A new study from a research team in Pittsburgh has suggested that patients that test positive for a type of autoantibody known as ‘anti-Th/To antibodies’ are at higher risk of developing PH over a 10-year timeframe in comparison to other patients. Anti-Th/To antibodies are rare and are only detected in around 5% of those with systemic sclerosis. More often than not, these are patients with limited cutaneous systemic sclerosis (those who have limited skin thickening) and Sine Scleroderma (who experience no skin symptoms).

Testing for these antibodies in these groups of patients could therefore help identify those at risk of scleroderma-related PH, allowing an appropriate clinical monitoring strategy to be put in place. Equally, for those at a lower level of risk it could minimise worry regarding treatment and monitoring of symptoms.

The study, led by Dr Shashank Suresh of the University of Pittsburgh Medical Center, included 204 patients referred to the Pittsburgh Scleroderma Center between 1980 and 2015 who tested positive for anti-Th/To antibodies. After adjusting for age and gender, testing positive for anti-Th/To antibodies was associated with a 3.3-fold increased risk of developing all-type PH within 10-years of the patient’s initial visit. Interestingly, the study also suggested that these patients are at a slightly greater risk of developing ILD over the same timeframe in comparison to other patients too.

Conversely however, whilst these patients had an increased risk of PH, the study also found them to fare better with symptoms of joint and tendon involvement – in fact, patients that tested positive for anti-Th/To antibodies had a 30% lower incidence of these symptoms in comparison to the control group of systemic sclerosis patients.

Potential Outcomes: Risk Stratification, Early Detection, & Reducing Uncertainty

The ability to accurately stratify patients into groups according to their risk of developing certain complications is a holy grail of modern scleroderma research. To achieve this, tests for autoantibodies like Th/To could eventually be incorporated into larger diagnostic tests which could more accurately pinpoint an individual’s risk of developing a complication such as PH.

Such testing in the early stages of systemic sclerosis would allow the development of ‘risk scores’ for certain complications associated with the condition. Those who are deemed to be at a higher risk of certain complications could be monitored more regularly by the appropriate healthcare professionals, enabling earlier detection and intervention with treatments to give the best possible outcomes for patients. Equally, patients could also benefit from an ability to live with less uncertainty about the likely progression of their condition. For example, patients with anti-Th/To antibodies could soon face reduced anxiety about developing joint and tendon problems over time. Research of this sort may not only allow the earlier detection of complications like PH, but also has the potential to reduce anxiety about the course of scleroderma – a source of worry which can affect patients’ mental health and quality of life.

Therefore, thanks to amazing projects such as the study carried out in Pittsburgh, people living with scleroderma will hopefully soon begin to experience the tangible benefits of research into the biomarkers for scleroderma and its related health issues. What’s certain is that studies of this type have the power to revolutionise the treatment of the condition, and are absolutely central to improving the lives of those living with scleroderma in the future.